Long-term management of patients with end-stage lung diseases

Briganti, Domenica Federica; D’Ovidio, Frank

doi:10.1016/j.bpa.2017.07.007

Cited by 9 publications

(4 citation statements)

References 57 publications

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“…Lung disease is the principal cause of morbidity and mortality in patients with cystic fibrosis (CF) [1,2]. The fundamental cause of CF is the recessive mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene, which encodes a chloride/bicarbonate (Cl − and HCO 3 − ) anion channel [3,4].…”

Section: Introductionmentioning

confidence: 99%

Progression of Cystic Fibrosis Lung Disease from Childhood to Adulthood: Neutrophils, Neutrophil Extracellular Trap (NET) Formation, and NET Degradation

Khan

Ali

Sweezey

et al. 2019

Genes

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Genetic defects in cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene cause CF. Infants with CFTR mutations show a peribronchial neutrophil infiltration prior to the establishment of infection in their lung. The inflammatory response progressively increases in children that include both upper and lower airways. Infectious and inflammatory response leads to an increase in mucus viscosity and mucus plugging of small and medium-size bronchioles. Eventually, neutrophils chronically infiltrate the airways with biofilm or chronic bacterial infection. Perpetual infection and airway inflammation destroy the lungs, which leads to increased morbidity and eventual mortality in most of the patients with CF. Studies have now established that neutrophil cytotoxins, extracellular DNA, and neutrophil extracellular traps (NETs) are associated with increased mucus clogging and lung injury in CF. In addition to opportunistic pathogens, various aspects of the CF airway milieux (e.g., airway pH, salt concentration, and neutrophil phenotypes) influence the NETotic capacity of neutrophils. CF airway milieu may promote the survival of neutrophils and eventual pro-inflammatory aberrant NETosis, rather than the anti-inflammatory apoptotic death in these cells. Degrading NETs helps to manage CF airway disease; since DNAse treatment release cytotoxins from the NETs, further improvements are needed to degrade NETs with maximal positive effects. Neutrophil-T cell interactions may be important in regulating viral infection-mediated pulmonary exacerbations in patients with bacterial infections. Therefore, clarifying the role of neutrophils and NETs in CF lung disease and identifying therapies that preserve the positive effects of neutrophils, while reducing the detrimental effects of NETs and cytotoxic components, are essential in achieving innovative therapeutic advances.

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Section: Introductionmentioning

confidence: 99%

Progression of Cystic Fibrosis Lung Disease from Childhood to Adulthood: Neutrophils, Neutrophil Extracellular Trap (NET) Formation, and NET Degradation

Khan

Ali

Sweezey

et al. 2019

Genes

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“…We designed a systematic review protocol based on the methodology outlined in the Center for Reviews and Dissemination handbook [17] and followed the PRISMA guidelines for data reporting [18]. The protocol is registered in the PROSPERO database (REGISTRATION NUMBER REMOVED IN ORDER NOT TO REVEAL THE AUTHORS4 IDENTITY) [19].…”

Section: Methodsmentioning

confidence: 99%

Correlates and outcomes of alcohol use after single solid organ transplantation: A systematic review and meta-analysis

Dobbels

Denhaerynck

Klem

et al. 2019

Transplantation Reviews

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Background: Reviews on alcohol use in transplant recipients focus on liver recipients and their risk of post-transplant rejection, but do not assess alcohol use in kidney, heart, or lung transplant recipients. This systematic review and meta-analysis aims to synthesize the evidence on correlates and outcomes of any alcohol use and at-risk drinking after solid organ transplantation (Tx). Methods: We searched 4 databases for quantitative studies in adult heart, liver, kidney and lung Tx recipients, investigating associations between post-Tx alcohol use and correlates and/or clinical, economic or quality of life outcomes. Paper selection, data extraction and quality assessment were performed by 2 reviewers independently. A pooled odds ratio (OR) was computed for each correlate/outcome reported ≥ 5 times. Results: Of the 5,331 studies identified, 76 were included in this systematic review (93.3% on liver Tx; mean sample size 148.9 (SD= 160.2); 71.9% male; mean age 48.9 years (SD= 6.5); mean time post-Tx 57.7 months (SD= 23.1)). On average, 23.6% of patients studied used alcohol post-transplant. Ninety-three correlates of any post-Tx alcohol use were identified, and 9 of the 19 pooled ORs were significantly associated with a higher odds for any post-Tx alcohol use: male gender, being employed post-transplant, smoking pre-transplant, smoking post-transplant, a history of illicit drug use, having first-degree relatives who have alcohol-related problems, sobriety < 6 months prior to transplant, a history of psychiatric illness, and having received treatment for alcohol-related problems pre-transplant. On average 15.1% of patients had at-risk drinking. A pooled OR was calculated for 6 of the 47 correlates of post-Tx at risk drinking investigated, of which pre-transplant smoking was the only correlate being significantly associated with 2 this behavior. None of the outcomes investigated were significantly associated with any use or at-risk drinking. Conclusion: Correlates of alcohol use remain under-investigated in solid organ transplant recipients other than liver transplantation. Further research is needed to determine whether any alcohol use or atrisk drinking is associated with poorer post-transplant outcomes. Our meta-analysis highlights avenues for future research of higher methodological quality and improved clinical care.

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“…In fact, a recent study highlighted the lack of validated NIV initiation criteria for people with CF. 17 It is also unclear whether the decision to implement NIV as an adjunct to treatment is made by the physical therapist, or by other members of the multidisciplinary team. Three previous survey studies have explored NIV use in the CF population.…”

Section: Introductionmentioning

confidence: 99%

Clinical usage of non-invasive ventilation by physical therapists in cystic fibrosis centres across Australia: A cross-sectional survey study

Foxcroft

Chambers

Cobb

et al. 2021

Preprint

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BACKGROUND: This study investigated clinical usage of non-invasive ventilation during physical therapy for people with cystic fibrosis. Specific research questions were: 1. What are the clinical indications, contraindications and patient selection criteria for non-invasive ventilation use as an adjunct to physical therapy in people with cystic fibrosis? 2. Who implements non-invasive ventilation, what settings are used and how are they determined? 3. What outcome measures are used to determine the effectiveness of non-invasive ventilation as an adjunct to physical therapy and what are the main benefits and complications? METHODS: A purpose-designed survey was sent to 23 Australian cystic fibrosis centres. RESULTS: Fifteen centres (65%) responded, with 13 reporting current utilization of non-invasive ventilation to assist physical therapy. Non-invasive ventilation was most commonly (85%) used in patients with lung function <40% predicted. Physical therapy clinical indications included shortness of breath at rest (100%) and during airway clearance (100%), and fatigue during airway clearance (100%). Physical therapists were responsible for initiating non-invasive ventilation (62%), setting up (85%) and determining settings (62%). Bi-level ventilation was the only chosen ventilation mode. Benefits reported included improved ease of airway clearance (100%), reduced fatigue (92%) and decreased dyspnoea (85%). Only one complication of haemoptysis was reported. CONCLUSIONS: Non-invasive ventilation was used during physical therapy in people with cystic fibrosis who had severe disease, mostly during airway clearance to improve tolerability of treatment. Australian physical therapists initiated non-invasive ventilation when people with cystic fibrosis experienced shortness of breath or fatigue during treatment, aligning with current clinical guidelines. Clinical usage was largely consistent across centres, with numerous benefits and few complications reported. Further research is required to explore benefits of non-invasive ventilation use during physical therapy. Keywords: Cystic Fibrosis, Noninvasive Ventilation, Bilevel Positive Airway Pressure, Physical Therapy, Airway Clearance, Exercise

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Long-term management of patients with end-stage lung diseases

Cited by 9 publications

References 57 publications

Progression of Cystic Fibrosis Lung Disease from Childhood to Adulthood: Neutrophils, Neutrophil Extracellular Trap (NET) Formation, and NET Degradation

Progression of Cystic Fibrosis Lung Disease from Childhood to Adulthood: Neutrophils, Neutrophil Extracellular Trap (NET) Formation, and NET Degradation

Correlates and outcomes of alcohol use after single solid organ transplantation: A systematic review and meta-analysis

Clinical usage of non-invasive ventilation by physical therapists in cystic fibrosis centres across Australia: A cross-sectional survey study

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